Scenesse for Erythropoietic Protoporphyria

SCENESSE is a medicine that contains the active substance afamelanotide. Afamelanotide is a synthetic form of a body hormone called alpha-melanocyte stimulating hormone (α-MSH). Afamelanotide works like the natural hormone, helping skin cells produce eumelanin, which is a brown-black type of melanin pigment in the body.

Afamelanotide is used to increase the tolerance to sunlight in people with Erythropoietic Protoporphyria (EPP). This disease casues increased sensitivity to sunlight, resulting in prolonged pain and burning of the skin. Patients with EPP cannot stay out during daylight hours for any appreciable period, isolating and confining them to indoor living. By increasing the amount of eumelanin, SCENESSE can help to delay the onset of pain due to skin photosensitivity (sensitive to sunlight) and prolong the time spent outdoors, thus enriching life!

Patients with EPP may also benefit from cimetidine, an over-the-counter medicine labeled for the treatment of stomach ulcers, and Heliocare, an oral sun protectant made from a fern native to South America. It has been reported that 800mg of cimetidine (200mg x4) with breakfast and dinner will help increase tolerance for sunlight. It appears to work by blocking the pathway that produces the chemical that accumulates in the skin and causes the pain. It is very well-tolerated. Patients have reported less pain when taking Heliocare daily, combined with sun-protective clothing and sunscreens containing Helioplex or mexoryl. The newer physical sunscreens, which simply reflect the UV light are even better. Beta-carotene has also been reported to be of help in EPP. Beta-carotene has also been reported to be of help in EPP.

How is SCENESSE used?

The implant is placed under your skin using a catheter tube and hollow trocar (sharp-tipped needle-like device). The skin will be numbed where the implant is to be inserted. The implant is inserted directly under the skin folds on your waist or abdomen over your hip. At the end of the insertion procedure, you may be able to feel the implant under your skin. Over time the implant will be absorbed by the body, this will happen within 50 to 60 days after implantation.

One implant is placed every 2 months, not exceed 6 per year. The implant may be removed by a simple surgical procedure if needed.

Sun protection

Do not change the sun protection measures you normally follow to manage your disease and according to your skin phototype (UV sensitivity). Keep in mind that increased exposure to UV light will contribute to skin cancer development.

Your regimen should include sun-protective clothing, hats, shades and sunscreens with at least SPF 100. Zinc oxide is the gold standard for a sun block and should be used as well. Heliocare, an oral sun protectant, is a great adjunct to any sun protection regimen.

Because this medicine increases eumelanin, in most treated patients the skin will darken. This is an expected response to this medicine, and the darkening will slowly fade unless another implant is used.

Your doctor will need to regularly check your skin (full body) to monitor changes in moles (e.g. darkening) or other skin abnormalities. This is recommended at every implant appointment.

Please inform your doctor about new or changing skin growths. Arrange for an early appointment if change occurs.

Scenesse is a medicine that can give sufferers of these diseases a new lease on life. Because it was just approved by the FDA, it will require a lot of flips and twists to get it to the patients who need it.

The FDA approval of Scenesse for EPP in October 2019 was a watershed moment for US patients with EPP. It has been a long time coming and it is now ready for use. The path to insurance approval is long and arduous but doable. Because it was just approved by the FDA, it will require a lot of flips and twists to get it to the patients who need it. The path starts here by establishing your account with Klara. You will then contact us to set up your EPP consultation. A small silver lining to the Corona Virus epidemic has made it easier for us to help patients who do not live in Florida or Louisiana, where Dr Resnik is licensed.